Suspected Hirschsprung's-associated enterocolitis (HAEC) Treatment Guideline...
Papers by Pastor et al and Gosain et al outline frameworks and clinical guidelines for management of enterocolitis
Cites a guidelineMeera Kotagal0:21
The guideline is most appropriate for patients with either known or suspected Hirschsprung's who present with GI symptoms and/or fever
Cites a guidelineMeera Kotagal0:21
GI symptoms in HAEC include abdominal distension, vomiting, no or minimal stool, foul smelling stool, or explosive diarrhea
Systemic signs of concern include fever, lethargy, age-adjusted tachycardia, tachypnea, hypotension, or oliguria
Patients should be seen and evaluated as soon as possible, ideally within one hour by someone with clinical expertise such as a surgical fellow or attending
Cites a guidelineMeera Kotagal2:00
The exam should include a rectal exam
Cites a guidelineMeera Kotagal2:00
If the patient is less than four weeks out from surgery, fellows should discuss the rectal exam with an attending surgeon prior to performing it
Cites a guidelineMeera Kotagal2:00
Irrigations should not be delayed for patients to get an x-ray
Cites a guidelineMeera Kotagal2:00
Irrigations should be repeated as frequently as every eight hours, but even every six or four hours as needed for sicker patients
Cites a guidelineMeera Kotagal2:00
Abdominal films should be obtained upon arrival and then repeated again after an irrigation to demonstrate adequate decompression
Cites a guidelineMeera Kotagal3:00
Abdominal films can be repeated throughout the course of the hospitalization as clinically necessary
Cites a guidelineMeera Kotagal3:00
Patients should be NPO and started on IV fluids to assist with resuscitation and hydration
Cites a guidelineMeera Kotagal3:00
Patients without systemic signs who are mostly clinically well can be maintained on either IV or oral flagyl during hospitalization
Cites a guidelineMeera Kotagal3:00
All patients who are vomiting should be on IV antibiotics
Cites a guidelineMeera Kotagal3:00
Patients with systemic signs who are sicker need broad spectrum antibiotics, specifically Zosyn and flagyl at Cincinnati Children's
Cites a guidelineMeera Kotagal3:00
All patients should get a CBC and a basic metabolic panel
Cites a guidelineMeera Kotagal4:00
A venous blood gas should be obtained for sicker patients to help evaluate resuscitation needs
Cites a guidelineMeera Kotagal4:00
Patients with systemic signs should be evaluated for potential admission to the ICU
Cites a guidelineMeera Kotagal4:00
After an admission for enterocolitis, patients are continued on metronidazole and irrigations for a few weeks and then slowly tapered
Cites a guidelineMeera Kotagal5:00
Underlying anatomic issues such as a stricture or a transition zone pull through must be addressed to prevent recurrent enterocolitis
Colorectal Quiz Episode 19: Hirschsprung Disease - The Obstructed Patient Part 1
Post-pull-through obstruction can be caused by stricture, twist (any pull-through type), Soave cuff (Soave procedure), Duhamel spur or non-functional distended segment (Duhamel procedure), or non-functional segment (Rehbein procedure).
In obstructed Hirschsprung patients, the colon fills with liquid stool with severe bacterial overgrowth, causing fluid loss into the bowel lumen, hypovolemia, and bacterial translocation/bacteremia, all occurring without passage of stool.
Enterocolitis can occur before surgery, after surgery, and even after successful surgery in babies who don't relax their sphincters and hold stool so efficiently they develop enterocolitis.
For a patient with prior Hirschsprung diagnosis presenting sick, assume Hirschsprung-associated enterocolitis; treatment with rectal irrigations, IV fluids, and antibiotics will not cause harm even if the diagnosis is different (e.g., Crohn's disease, E. coli enterocolitis).
Post-pull-through enterocolitis within the first 3 months occurs in about 20% of patients, based on a study from Cincinnati and Columbus.
Families should not undergo Hirschsprung pull-through surgery until they can demonstrate ability to perform rectal irrigations, and they should be discharged with supplies to perform irrigations at home.
Cites a guidelineJason Frischer14:58
In rare circumstances where a patient is too ill and sedation in the ER doesn't work, go to the OR under general anesthesia for irrigation until the patient improves; occasionally an ileostomy is needed to get the child out of trouble and work up the pull-through problem later.
On contrast enema, it is important to look at the presacral space (space between the hollow of the sacrum and the pull-through); a widened presacral space is an abnormal finding.
On contrast enema, if the catheter is inserted too high or the balloon is under too much pressure, distal pathology cannot be easily ascertained.
A lateral view on contrast enema is important because it provides significant information about the presacral space and distal rectum anatomy.